The Prevalence and Clinical Spectrum of Primary Biliary Cirrhosis in a Defined Population

Abstract

Patients (33) with primary biliary cirrhosis representative of a well-defined population (240,000) during the decade 1973-82 were studied. Mean annual incidence was 13.7/106 and point prevalence, 92/106 inhabitants in 1982. An accumulation of asymptomatic cases, constituting 45% of all patients, with a normal life expectancy accounted for this high prevalence. During the study period no disease progress was seen in asymptomatic patients, in contrast to a 50% mortality in the symptomatic group. Disease progress in the latter group was reflected by deterioration of N-demethylating capacity and increasing bilirubin levels. Although an increasing prevalence of primary biliary cirrhosis was confirmed, the mortality rate during the study period was almost identical to that in an earlier period, 1951-60.